Ngaba nawe unesifo sikaGaucher? Makhe sithethe ngesiyobisi iTaliglucerase Alfa?

Ngamanye amaxesha sifumana izifo esingenakuzicinga, akunjalo? Isifo sikaGaucher naso sisifo esingaqhelekanga, kodwa kufuneka silawulwe ngokufanelekileyo. Ngoko ke ukuba wena okanye umntu osondeleyo kuwe unale meko, namhlanje siza kuthetha ngeyeza iTaliglucerase alfa, eliyinyango layo. Masiqonde nje ukuba yintoni, isebenza njani, kwaye yintoni ekufuneka uyilumkele.

Masiqale sibone ukuba yintoni iGaucher's Disease.

Ngamafutshane, isifo seGaucher sisifo esenzeka ngenxa yezinto ezithile zemfuza emzimbeni wethu. Kukho iintlobo ezininzi zee-enzyme ezenza umsebenzi obalulekileyo emzimbeni wethu. Kwesi sifo, i-enzyme ebizwa ngokuba yi -glucocerebrosidase ayiveliswanga kakuhle kwiiseli zomzimba wethu, okanye umsebenzi wayo uyancitshiswa. Le enzyme iqhekeza ezinye izinto ezinamafutha emzimbeni wethu kwaye yenze izinto eziluncedo kuzo. Ngoko ke xa le enzyme incipha, ezo zinto zinamafutha ziqala ukuqokelelana kwezinye iindawo zomzimba wethu, umzekelo , umongo wethambo, i-spleen, nesibindi . Xa ziqokelelana, kuvela iimpawu ezahlukeneyo. Oko kukuthi, abanye abantu banokuba neentlungu zamathambo, i-anemia, kunye ne-spleen ekhulisiweyo. Nangona oku kunokubonakala kunzima kancinci, akuyonto inkulu xa kuqondwa kakuhle.

Ngoko ke yintoni le yeza ibizwa ngokuba yiTaliglucerase Alfa?

I-Taliglucerase alfa liyeza elinikwa abantu abanesifo seGaucher, esithethe ngaso ngaphambili, ngokwenza i-enzyme abasweleyo emizimbeni yabo. Ngokuchanekileyo, le yindlela eyenziwe ngumntu ye-enzyme . Isetyenziselwa ukulawula iimpawu zesifo seGaucher. Kodwa kufuneka ukhumbule ukuba eli ayiloyeza eliza kunyanga ngokupheleleyo esi sifo . Kodwa linceda ukulawula iimpawu kwaye lenze ubomi bube lula kakhulu.

Ngamanye amaxesha oogqirha banokusebenzisa eli yeza kwezinye izinto, ngoko ke ukuba unemibuzo, kungcono ubuze ugqirha wakho okanye ingcali yezemithi.

Uya kulibona eli yeza phantsi kwegama lebrendi elithi ELELYSO .

Ndimele ndixelele iqela lezonyango ntoni ngaphambi kokuba ndisebenzise eli yeza?

Ngaphambi kokuba unikwe eli yeza, iTaliglucerase alfa, iqela lakho lezonyango (iqela lokhathalelo) kufuneka liqaphele izinto ezithile. Kuba, ngokusekelwe kolo lwazi, banokukunika unyango ngokukhuselekileyo. Masibone ukuba zeziphi:

• Ukuba wakha waba ne-allergy engaqhelekanga kwi-Taliglucerase alfa, amanye amayeza, ukutya, iidayi zokutya, okanye izithinteli, ngokuqinisekileyo kufuneka usixelele. Oku kungabonakala ngathi yinto encinci, kodwa kubaluleke kakhulu.
• Ukuba ukhulelwe, okanye ukuba uceba ukukhulelwa kungekudala, yazisa ugqirha wakho.
• Ukuba ungumama oncancisayo, ungalibali ukukhankanya loo nto.

Ngokulungisa ezi zinto, iqela lezonyango linokuceba unyango olufanelekileyo nolukhuselekileyo kuwe.

Ndingakunika njani eli yeza?

Eli yeza linikwa ngokulifaka emthanjeni . Ngamafutshane, linikwa emthanjeni. Oku kudla ngokwenziwa esibhedlele okanye ekliniki yingcali yezempilo eqeqeshwe ukulinika. Oku kuthetha ukuba awunakulisebenzisa ekhaya. Leyo yindlela yokuzikhusela.

Ngaba eli yeza lifanele linikwe abantwana abancinci?

Ukuba umntwana wakho usebenzisa eli yeza, iTaliglucerase alfa, ngokuqinisekileyo kuya kufuneka uthethe nogqirha wakho wabantwana ngalo. Kuba ngamanye amaxesha kufuneka kuthathwe ingqalelo ekhethekileyo xa kunikwa abantwana abancinci olu hlobo lweyeza. Izinto ezifana nomthamo kunye neshedyuli yokusetyenziswa kufuneka zilungiswe ukuze zilungele bona.

Kuza kwenzeka ntoni ukuba uthatha amayeza amaninzi kakhulu?

Khumbula, ukuba ucinga ukuba unikwe eli yeza lininzi kakhulu, okanye ukuba ukrokrela ukuba ulinikwe, kufuneka ufowunele iziko lokulawula ityhefu ngokukhawuleza okanye igumbi likaxakeka lesibhedlele elikufutshane . Kwimeko ezinje, ukuthatha inyathelo ngokukhawuleza yeyona nto ibalulekileyo.

Nceda uqaphele: Eli yeza limiselwe wena kuphela. Musa ukulinika umhlobo okanye ilungu losapho, nokuba baneempawu ezifanayo. Iyeza elifanelekileyo kunye nomlinganiselo unokwahluka kumntu ngamnye.

Yintoni omawuyenze ukuba uphoswe yidosi yeyeza?

Kubaluleke kakhulu ukusela eli yeza ngexesha ngaphandle kokulitsiba idosi. Oku kungenxa yokuba uza kufumana inzuzo enkulu kwiyeza kuphela xa ulisela. Ukuba awukwazi ukuya esibhedlele/eklinikhi ukuze ufumane unyango ngosuku olucwangcisiweyo, fowunela ugqirha wakho okanye ingcali yezempilo ngoko nangoko uze ubazise . Baza kukuxelela into omawuyenze emva koko.

Ziziphi ezinye izinto ezinokusebenzisana nale mithi?

Ngokuqhelekileyo kuthiwa akukho ntsebenziswano inzulu elindelekileyo phakathi kwesi siyobisi seTaliglucerase alfa kunye nezinye iziyobisi. Nangona kunjalo, oko akuthethi ukuba asiyi kusebenzisana nantoni na. Ngamanye amaxesha, iziphumo ebezingalindelekanga ezincinci zinokwenzeka.

Ngoko ke, nika ugqirha wakho uluhlu olupheleleyo lwamayeza owasebenzisayo – kungekuphela nje lawo anikwe ngugqirha, kodwa nawaphi na amayeza emifuno, amayeza angagunyaziswanga ngugqirha, okanye izongezo zokutya ezifana neevithamini. Kwakhona, ukuba uyatshaya, usela utywala, okanye usebenzisa iziyobisi ezingekho mthethweni.Thembeka nakwelo tyala, kuba ezinye izinto zinokuba nemiphumo emibi xa zidityaniswe namayeza akho.

Yintoni omele uyilumkele xa usebenzisa eli yeza?

Ndwendwela ugqirha wakho ukuze uhlolwe rhoqo ukuze ubone ukuba unyango lwakho luqhubeka njani. Oku kubaluleke kakhulu. Ugqirha wakho uza kukujonga ukuba amayeza ayasebenza na kwaye iimpawu zakho ziya zisiba ngcono na. Ukuba iimpawu zakho aziphucuki, okanye ziya zisiba mandundu , xelela ugqirha wakho okanye ingcali yezempilo. Musa ukugcina nantoni na kuwe.

Zithini iziphumo ebezingalindelekanga ezinokubakho zale yeza?

Njengayo nayiphi na iyeza, i-Taliglucerase alfa inokubangela ezinye iziphumo ebezingalindelekanga. Asinguye wonke umntu ozifumanayo, kodwa kulungile ukuzazi. Ezinye zinzima kwaye zifuna unyango olukhawulezileyo. Ezinye ziqhelekile kwaye ziya kuphela kwiintsuku ezimbalwa. Makhe sizijonge.

Iziphumo ebezingalindelekanga ekufuneka zixelwe kugqirha okanye kwingcali yezempilo ngoko nangoko:

Ukuba ufumana naziphi na kwezi mpawu, kubalulekile ukufuna ingcebiso kagqirha ngokukhawuleza ngaphandle kokulibazisa, njengoko ngamanye amaxesha ezi zinokuba ziimpawu zesifo esinzulu ngakumbi:

• Iimpendulo ze-allergy : Ezi ziquka ukurhawuzelela kwesikhumba ngequbuliso, ukurhawuzelela okanye ukurhawuzelela okukhulu, ukudumba kobuso, imilebe, okanye ulwimi. Ezi zinokubangela ubunzima bokuphefumla.
• Iinzipho okanye imilebe eluhlaza okwesibhakabhaka .
• Iingxaki zokuphefumla , ukuziva ngathi uyafuthaniseka.
• Intlungu yesifuba okanye ukuziva uxinezelekile esifubeni.
• Ukukhwehlela okungenakunqandwa .
• Ukubetha kwentliziyo okukhawulezayo okanye okungahambelaniyo ngequbuliso .
• Ububomvu bobuso, ubushushu bomzimba kunye nokubila (ukugabha) .
• Uxinzelelo lwegazi oluphantsi , oko kuthetha ukuba uzive unesizungu kwaye ukhathazekile.
• Intlungu, ukubomvu, okanye ukurhawuzelelwa kwindawo apho kufakwe khona inaliti , kwaye ngamanye amaxesha kunokudumba.
• Ukudumba kwamaqatha , iinyawo, izandla.

Iziphumo ebezingalindelekanga ezihlala zingadingi nyango, kodwa kufuneka zixelwe kugqirha ukuba ziyaqhubeka okanye ziyakhathaza:

Ezi zinto zihlala ziphela emva kweentsuku ezimbalwa, kodwa ukuba ziyaqhubeka, okanye ziyakukhathaza kangangokuba zingakuvumeli ukuba wenze imisebenzi yakho yemihla ngemihla, xelela ugqirha wakho:

• Ukuziva ubanda, uqhaqhazela (ukubanda) .
• Urhudo .
• Ifiva .
• Intloko ebuhlungu .
• Isicaphucaphu, ukuhlanza .
• AyinyamezelekiNdiziva ndidiniwe kakhulu.
• Isisu esibuhlungu, njengokungagaywa kakuhle kokutya .

Olu luhlu aluzizo zonke iziphumo ebezingalindelekanga. Ukuba ubona nantoni na engaqhelekanga okanye utshintsho emzimbeni wakho, nceda udibane nogqirha wakho. Musa ukuthatha nantoni na lula.

Uligcina phi eli yeza?

Ekubeni eli yeza liza kunikwa esibhedlele okanye ekliniki, akuyi kufuneka uligcine ekhaya . Oku kuthetha ukuba akufuneki ukhathazeke malunga nokuba iyeza liza konakala okanye liza kugcinwa ngokufanelekileyo na. Loo mbopheleleko isemagxeni abasebenzi besibhedlele okanye bekliniki.

Okokugqibela, zimbalwa izinto ekufuneka uzikhumbule:

I-Taliglucerase alfa liyeza elibalulekileyo elinceda ukulawula iimpawu zesifo sikaGaucher. Oku kuquka ukutshintsha ngokungekho mthethweni i-enzyme engekho emzimbeni. Eli yeza linikwa njengenaliti emthanjeni, esibhedlele. Qiniseka ukuba uxelela ugqirha wakho ukuba une-allergies, ukhulelwe, okanye uthatha amanye amayeza. Iziphumo ebezingalindelekanga zinokwenzeka, ngoko ke funa ingcebiso yezonyango ngokukhawuleza ukuba ufumana naziphi na iimpawu ezinzulu . Kubaluleke kakhulu ukufumana unyango ngexesha kwaye uhlale unxibelelana nogqirha wakho.

Musa ukoyika ukubuza ugqirha wakho, ikhemisti, okanye umboneleli wezempilo nayiphi na imibuzo okanye iinkxalabo onokuba nazo. Bakho ukuze bakuncede. Impilo yakho ibalulekile nakubo.

I- Taliglucerase alfa, Isifo sikaGaucher, i-enzyme, amayeza, iziphumo ebezingalindelekanga